Mental Health Care for Children with Heart Disease and Their Families: Practical Approaches and Considerations for the Pediatric and Pediatric Cardiology Clinician.

Mental health conditions are a common comorbidity among children living with heart disease. Children with congenital heart disease are more likely to have a mental health condition than their unaffected peers or peers with other chronic illnesses, and mental health risk persists across their lifetime. While poorer mental health in adults with congenital heart disease is associated with worse overall health outcomes, the association between mental health and cardiac outcomes for children with heart disease remains unknown. Despite this, it is suspected that mental health conditions go undiagnosed in children with heart disease and that many affected children and adolescents do not receive optimal mental health care. In this article, we review mental health in congenital heart disease across the lifespan, across domains of care, and across diagnoses. Further directions to support mental health care for children and adolescents with heart disease include practical screening and access to timely referral and mental health resources.

Disparities in resource utilisation by families of children with cardiac conditions.

OBJECTIVES: There are limited data documenting sources of medical information that families use to learn about paediatric cardiac conditions. Our study aims to characterise these resources and to identify any disparities in resource utilisation. We hypothesise there are significant variations in the resources utilised by families from different educational and socio-economic backgrounds. METHODS: A survey evaluating what resources families use (websites, healthcare professionals, social media, etc.) to better understand paediatric cardiac conditions was administered to caretakers and paediatric patients at Morgan Stanley Children's Hospital. Patients with a prior diagnosis of CHD, cardiac arrhythmia, and/or heart failure were included. Caretakers' levels of education (fewer than 16 years vs. 16 years or more) and patients' medical insurance types (public vs. private) were compared with regard to the utilisation of resources. RESULTS: Surveys completed by 137 (91%) caretakers and 27 (90%) patients were analysed. Websites were utilised by 72% of caretakers and 56% of patients. Both private insurance and higher education were associated with greater reported utilisation of websites, healthcare professionals, and personal networks (by insurance p = 0.009, p = 0.001, p = 0.006; by education p = 0.022, p < 0.001, p = 0.018). They were also more likely to report use of electronic devices (such as a computer) compared to those with public medical insurance and fewer than 16 years of education (p < 0.001, p < 0.001, respectively). CONCLUSION: Both levels of education and insurance status are associated with the utilisation of informative resources and digital devices by families seeking to learn more about cardiac conditions in children.

3D-Printed Cardiac Models for Fetal Counseling: A Pilot Study and Novel Approach to Improve Communication.

A fetal cardiology consultation involves using two-dimensional drawings to explain the cardiac anatomy which can result in inherent variation in how the congenital heart disease (CHD) is conveyed. In this pilot study, we incorporated three-dimensional printed (3DP) models into fetal counseling to demonstrate feasibility and evaluate the impact on parental knowledge, understanding, and anxiety. Parents with a prenatal diagnosis of a muscular ventricular septal defect (VSD) and/or coarctation of aorta were enrolled. Providers were randomized into a Model or Drawing Group and crossed after six months. Parents completed a survey after the consultation which evaluated knowledge of the CHD lesion, expectant surgical management, self-rated understanding, attitude towards the visualization tool, and anxiety. Twenty-nine patients enrolled over a 12 month period. Twelve consultations were done for coarctation of aorta, 13 for VSD, and four for coarctation with a VSD. Both Model and Drawing groups scored similarly in self-reported understanding and confidence, helpfulness of and improvement in communication with the visualization tool. The Model group had higher scores on questions related to the CHD anatomy and surgical intervention [5 [4-5] versus 4 [3.5-5]], p = 0.23 although this didn't reach statistical significance. For the majority (83%) of consultations, the cardiologist agreed that the 3D model improved communication. In this pilot study, we demonstrate the use of 3DP cardiac models during prenatal CHD counseling is feasible and produces results related to parental understanding and knowledge that are equal to and possibly better than the current standard of care.

Minimum Supervision Levels Required for Pediatric Cardiology Fellowship Graduation by Pediatric Cardiology Fellowship Program Directors.

Entrustable professional activities (EPAs) are "observable essential tasks expected to be performed by a physician for safe patient care in practice." Six Pediatric Cardiology (PC) EPAs and their level of supervision (LOS) scales were developed by medical educators in PC using a modified Delphi process and reviewed by the Subspecialty Pediatrics Investigator Network (SPIN). However, their general use in assessment for PC fellows for graduation requirements has yet to be studied. The objective of this study was to determine the minimum LOS required for PC fellows to graduate and compare it with the minimum LOS expected for safe and effective practice for the six PC EPAs, from the perspective of the PC Fellowship Program Directors(FPD). All Fellowship Program Directors(FPD) of ACGME-accredited PC fellowships were surveyed through SPIN between April 2017 and August 2017. For each of the PC EPAs, the FPDs were asked to indicate the minimum LOS expected for graduation and whether they would allow a fellow to graduate if this level was not achieved and the minimum LOS expected for a practicing pediatric cardiologist to provide safe and effective patient care. The minimum LOS was defined as the LOS for which no more than 20% of FPDs would want a lower level. The survey response rate was 80% (47/59). The majority of the FPDs did not require a minimum LOS of five corresponding to unsupervised practice in any of the six PC EPAs at graduation. For EPAs related to imaging, arrhythmia management, and management of cardiac problems, the minimum LOS for graduation was 3, corresponding to being "trusted to perform a task with indirect supervision for most simple and a few complex cases." For the EPAs related to interventional cardiology, heart failure pulmonary hypertension, and cardiac intensive care, the minimum LOS for graduation was 2, corresponding to being "trusted to perform a task only with direct supervision and coaching." The minimum LOS considered necessary for safe and effective practice for all but one EPA was 3. For the EPA related to the management of cardiac problems, the minimum LOS for safe practice was 4, corresponding to being "trusted to execute tasks independently except for few complex and critical cases." Most PC FPDs reported they would not require fellows to achieve the highest entrustment level for any of the six PC EPAs for graduation. It is crucial that educational programs evolve to address these essential activities during training better and that stakeholders ensure that graduating PC fellows have adequate resources and infrastructure to continue professional development as early career pediatric cardiologists.

Paediatrician's guide to post-operative care for biventricular CHD: a review.

IMPORTANCE: Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients. OBSERVATIONS: The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation. CONCLUSIONS AND RELEVANCE: Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Paediatrician's guide to post-operative care for functionally univentricular CHD: a review.

IMPORTANCE: Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care. OBSERVATIONS: We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician's approach to single ventricle patients at different stages of surgical palliation. CONCLUSIONS AND RELEVANCE: Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Loss of Consciousness in the Young Child.

In the very young child (less than eight years of age), transient loss of consciousness represents a diagnostic and management dilemma for clinicians. While most commonly benign, syncope may be due to cardiac dysfunction which can be life-threatening. It can be secondary to an underlying ion channelopathy, cardiac inflammation, cardiac ischemia, congenital heart disease, cardiomyopathy, or pulmonary hypertension. Patients with genetic disorders require careful evaluation for a cardiac cause of syncope. Among the noncardiac causes, vasovagal syncope is the most common etiology. Breath-holding spells are commonly seen in this age group. Other causes of transient loss of consciousness include seizures, neurovascular pathology, head trauma, psychogenic pseudosyncope, and factitious disorder imposed on another and other forms of child abuse. A detailed social, present, past medical, and family medical history is important when evaluating loss of consciousness in the very young. Concerning characteristics of syncope include lack of prodromal symptoms, no preceding postural changes or occurring in a supine position, after exertion or a loud noise. A family history of sudden unexplained death, ion channelopathy, cardiomyopathy, or congenital deafness merits further evaluation. Due to inherent challenges in diagnosis at this age, often there is a lower threshold for referral to a specialist.

Role of a Pediatric Cardiologist in the COVID-19 Pandemic.

Coronavirus disease 2019 (COVID-19) has affected patients across all age groups, with a wide range of illness severity from asymptomatic carriers to severe multi-organ dysfunction and death. Although early reports have shown that younger age groups experience less severe disease than older adults, our understanding of this phenomenon is in continuous evolution. Recently, a severe multisystem inflammatory syndrome in children (MIS-C), with active or recent COVID-19 infection, has been increasingly reported. Children with MIS-C may demonstrate signs and symptoms of Kawasaki disease, but also have some distinct differences. These children have more frequent and severe gastrointestinal symptoms and are more likely to present with a shock-like presentation. Moreover, they often present with cardiovascular involvement including myocardial dysfunction, valvulitis, and coronary artery dilation or aneurysms. Here, we present a review of the literature and summary of our current understanding of cardiovascular involvement in children with COVID-19 or MIS-C and identifying the role of a pediatric cardiologist in caring for these patients.

Adaptations of paediatric cardiology practice during the COVID-19 pandemic.

During the initial surge of the COVID-19 pandemic in the spring and summer of 2020, paediatric heart centres were forced to rapidly alter the way patient care was provided to minimise interruption to patient care as well as exposure to the virus. In this survey-based descriptive study, we characterise changes that occurred within paediatric cardiology practices across the United States and described provider experience and attitudes towards these changes during the pandemic. Common changes that were implemented included decreased numbers of procedures, limiting visitors and shifting towards telemedicine encounters. The information obtained from this survey may be useful in guiding and standardising responses to future public health crises.

Performing an urgent neonatal cardiac intervention safely during the COVID-19 pandemic.

The current pandemic has driven the medical community to adapt quickly to unprecedented challenges. Among these challenges is the need to minimize staff exposure to COVID-19 during neonatal cardiac procedures. In this report, we describe measures we have taken to protect health care workers while ensuring successful outcomes. These measures include wearing appropriate personal protective equipment, physical distancing, designating separate delivery and transport teams, and limiting the number of providers in direct contact with any patient who is infected or whose infection status is unknown. LEARNING OBJECTIVES: 1.To understand specific challenges caused by the COVID-19 pandemic for patients with congenital heart disease needing urgent neonatal intervention.2.To recognize measures that can be taken to minimize health care workers' exposures to the virus during high-risk neonatal cardiac procedures.3.To review the management of neonates with d-transposition of the great arteries and inadequate mixing.

Cardiac workup and monitoring in hospitalised children with COVID- 19.

Approximately, 1.7 million individuals in the United States have been infected with SARS-CoV-2, the virus responsible for the novel coronavirus disease-2019 (COVID-19). This has disproportionately impacted adults, but many children have been infected and hospitalised as well. To date, there is not much information published addressing the cardiac workup and monitoring of children with COVID-19. Here, we share the approach to the cardiac workup and monitoring utilised at a large congenital heart centre in New York City, the epicentre of the COVID-19 pandemic in the United States.

Neonatal Palliative Care for Complicated Cardiac Anomalies: A 10-Year Experience of an Interdisciplinary Program at a Large Tertiary Cardiac Center.

OBJECTIVES: To report the outcomes of a Neonatal Palliative Care (NPC) Program at a large tertiary cardiac center caring for a subset of fetuses and neonates with life-limiting cardiac diagnoses or cardiac diagnoses with medical comorbidities leading to adverse prognoses. STUDY DESIGN: The Neonatal Comfort Care Program at New York-Presbyterian Morgan Stanley Children's Hospital/Columbia University Medical Center is an interdisciplinary team that offers the option of NPC to neonates prenatally diagnosed with life-limiting conditions, including single ventricle (SV) congenital heart disease (CHD) or less severe forms of CHD complicated by multiorgan dysfunction or genetic syndromes. RESULTS: From 2008 to 2017, the Neonatal Comfort Care Program cared for 75 fetuses or neonates including 29 with isolated SV CHD, 36 with CHD and multiorgan dysfunction and/or severe genetic abnormalities, and 10 neonates with a prenatal diagnosis of isolated CHD and postnatal diagnoses of severe conditions who were initially in intensive care before transitioning to NPC because of a poor prognosis. CONCLUSIONS: At New York-Presbyterian Morgan Stanley Children's Hospital/Columbia University Medical Center, a large tertiary cardiac center, 13.5% of parents of fetuses or neonates with isolated SV CHD opted for NPC. Twenty-six of 29 newborns with SV CHD treated with NPC died. Of the remaining, 2 neonates with mixing lesions are alive at 3 and 5 years of age, and 1 neonate was initially treated with NPC and then pursued surgical palliation. These results suggest that NPC is a reasonable choice for neonates with SV CHD.

Utility of 3D Printed Cardiac Models for Medical Student Education in Congenital Heart Disease: Across a Spectrum of Disease Severity.

The most common modes of medical education for congenital heart disease (CHD) rely heavily on 2-dimensional imaging. Three-dimensional (3D) printing technology allows for the creation of physical cardiac models that can be used for teaching trainees. 3D printed cardiac models were created for the following lesions: pulmonic stenosis, atrial septal defect, tetralogy of Fallot, d-transposition of the great arteries, coarctation of the aorta, and hypoplastic left heart syndrome. Medical students participated in a workshop consisting of different teaching stations. At the 3D printed station, students completed a pre- and post-intervention survey assessing their knowledge of each cardiac lesion on a Likert scale. Students were asked to rank the educational benefit of each modality. Linear regression was utilized to assess the correlation of the mean increase in knowledge with increasing complexity of CHD based on the Aristotle Basic Complexity Level. 45 medical students attended the CHD workshop. Students' knowledge significantly improved for every lesion (p < 0.001). A strong positive correlation was found between mean increase in knowledge and increasing complexity of CHD (R2 = 0.73, p < 0.05). The 3D printed models, pathology specimens and spoken explanation were found to be the most helpful modalities. Students "strongly agreed" the 3D printed models made them more confident in explaining congenital cardiac anatomy to others (mean = 4.23, ± 0.69), and that they recommend the use of 3D models for future educational sessions (mean = 4.40, ± 0.69). 3D printed cardiac models should be included in medical student education particularly for lesions that require a complex understanding of spatial relationships.

Effectiveness of echocardiography simulation training for paediatric cardiology fellows in CHD.

Simulation is used in many aspects of medical training but less so for echocardiography instruction in paediatric cardiology. We report our experience with the introduction of simulator-based echocardiography training at Weill Cornell Medicine for paediatric cardiology fellows of the New York-Presbyterian Hospital of Columbia University and Weill Cornell Medicine. Knowledge of CHD and echocardiographic performance improved following simulation-based training. Simulator training in echocardiography can be an effective addition to standard training for paediatric cardiology trainees.

Drug-Coated Balloon Angioplasty: A Novel Treatment for Pulmonary Artery In-Stent Stenosis in a Patient with Williams Syndrome.

A 20-month-old boy with Williams syndrome had undergone multiple surgical and catheter-based interventions for resistant peripheral pulmonary arterial stenoses with eventual bilateral stent placement and conventional balloon angioplasty. He persistently developed suprasystemic right ventricular (RV) pressure. Angioplasty with a drug-coated balloon (DCB) was performed for in-stent restenosis and to remodel his distal pulmonary vessels bilaterally. This resulted in immediate improvement in the in-stent stenosis and resultant decrease in RV pressure. Follow-up catheterization two months later continued to show long-lasting improvement in the in-stent stenosis. We hypothesize that the anti-proliferative effects of DCBs may be of benefit in the arteriopathy associated with Williams syndrome. We report this as a novel use of a DCB in the pulmonary arterial circulation in a patient with Williams syndrome.

When Is It Better to Wait? Surgical Timing and Recurrence Risk for Children Undergoing Repair of Subaortic Stenosis.

Recurrence of subaortic stenosis (SAS) after surgery is common in children. The effects of patient characteristics and surgical timing on disease recurrence are largely unknown. We performed a retrospective study, assessing the relative effects of patient age and left ventricular outflow tract (LVOT) gradient on the need for reoperation for recurrent SAS. We included all children <20 years of age who underwent initial surgical resection of SAS at our center, January 2003-December 2013. Stratified logistic regression was performed, considering the effects of patient demographics, clinical characteristics, echocardiographic parameters, and operative technique, and clustering standard errors by surgeon. The multivariable model was used to simulate predicted probabilities of recurrent SAS for children at varying ages and baseline LVOT gradients. Sixty-three patients (38 males) underwent initial operation for SAS. Patients were followed for a median of 3.7 years (IQR 1.2-7.1). Twenty-one percent of patients (n = 13) underwent reoperation for SAS. Twelve were male. For every 10 mmHg increase in preoperative peak gradient in boys, the odds of reoperation for SAS doubled (OR 2.01, CI 1.5-2.72, p < 0.001), and for every additional 6 months of age, the odds of reoperation decreased by 14% (OR 0.86, CI 0.84-0.88, p < 0.001). Both younger age and higher preoperative outflow tract gradient are independently associated with risk of reoperation. Clinicians should consider the age and rate of LVOT gradient change-and not just the absolute gradient-in determining initial surgical timing.

Congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa: a case report.

The mitral-aortic intervalvular fibrosa is an area of fibrous continuity between the mitral and aortic valves. We present the first case of a congenital pseudoaneurysm in this region, detected prenatally as an isolated cardiac defect, which was followed-up conservatively postnatally. The diagnosis was confirmed by echocardiogram demonstrating blood flow into the pouch during systole and into the left ventricular outflow tract during diastole. The infant has been followed-up with serial echocardiograms demonstrating stable size and appearance of the lesion, without signs of obstruction, making close continued observation a reasonable approach.

Primary arterial switch operation as a strategy for total correction of Taussig-Bing anomaly: a 21-year experience.

BACKGROUND: Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the long-term outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly. METHODS AND RESULTS: A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011. Median age at arterial switch operation was 7 (range, 2-192) days, and median operative weight was 3.2 (1.4-6.2) kg. Aortic arch obstruction was present in 30 patients (70%). Hospital mortality was 7% (n=3). Follow-up was available for 37 hospital survivors at a mean of 8.1 (± 6.3) years. Late mortality was 2% (n=1). At follow-up, all patients were in New York Heart Association functional class I. Freedom from transcatheter or surgical reintervention was 73% at 1 year, 64% at 5 years, and 60% at 10 years. Eleven patients underwent 13 catheter reinterventions on the pulmonary arteries (n=8) or aortic arch (n=5). Seven patients underwent 11 reoperations, including relief of right ventricular outflow tract obstruction (n=5), pulmonary arterioplasty (n=3), recoarctation repair (n=2), and tricuspid valve repair (n=1). By multivariate analysis, a preoperative aortic valve annulus z score of ≤-2.5 was associated with reintervention (hazard ratio, 7.66 [95% confidence interval, 1.29-45.6], P=0.03). CONCLUSIONS: Although reintervention is common, primary correction of Taussig-Bing anomaly with arterial switch operation can be achieved in all patients with low mortality and good long-term outcomes.

Comparison of method of conception in fetuses undergoing echocardiography at a tertiary referral center.

OBJECTIVES: We compared the proportion of conception with and without in vitro fertilization (IVF) in fetuses with and without congenital heart disease (CHD). METHODS: This was a retrospective review of fetal echocardiograms at Columbia University from 2007 to 2010, to identify the mode of conception. RESULTS: Echocardiography was performed on 2828 fetuses, and 2761 (97.6%) had the method of conception documented. CHD was diagnosed in 22.4%, consisting predominantly of complex CHD. The proportion of IVF conception was lower in fetuses with CHD (6.9% CHD vs 10.3% no CHD, OR = 0.65 [95% CI 0.46-0.92], p = 0.01). IVF fetuses were conceived by elder mothers and were more likely part of a multiple gestation than those without IVF. In a multivariate model controlling for maternal age and multiple gestation, IVF was not associated with CHD diagnosis (OR = 1.1 [95% CI 0.77-1.7], p = 0.51). CONCLUSION: At a tertiary referral center, fetuses with CHD were not more likely to be conceived by IVF after controlling for maternal age and multiple gestation. These results differ from those of several previous reports, which may be related to our study population, and the exclusion of isolated atrial shunts and patent ductus arteriosus, which are normal fetal findings.